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Review
. 2023 Jan;482(1):99-112.
doi: 10.1007/s00428-022-03423-3. Epub 2022 Oct 10.

The international consensus classification of mastocytosis and related entities

Roos J Leguit  1 , Sa A Wang  2 , Tracy I George  3 , Alexandar Tzankov  4 , Attilio Orazi  5
Affiliations
Review

The international consensus classification of mastocytosis and related entities

Roos J Leguit et al. Virchows Arch. 2023 Jan.

Abstract

Mastocytosis is a neoplasm characterized by a clonal proliferation of mast cells, which accumulate in one or multiple organs, associated with an extremely heterogeneous clinical presentation. The disease can be limited to the skin (cutaneous mastocytosis) that is mostly seen in childhood and usually behaves in a benign fashion. Adult patients most often present with systemic disease with or without skin lesions. This includes indolent forms such as indolent systemic mastocytosis and its subvariant bone marrow mastocytosis, and smoldering systemic mastocytosis as well as aggressive forms including aggressive systemic mastocytosis, systemic mastocytosis with an associated myeloid neoplasm (previously called systemic mastocytosis with an associated hematologic neoplasm), and mast cell leukemia. In addition, mast cell sarcoma is a rare aggressive form of mastocytosis that can present in the skin as well as at extracutaneous sites. This review article focuses on the updates in mastocytosis of the 2022 international consensus classification (ICC).

Keywords: Aggressive mastocytosis; Bone marrow biopsy; ICC; Mast cell leukemia; Mast cell sarcoma; Mastocytosis.

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References

    1. Tanaka S, Furuta K (2021) Roles of IgE and histamine in mast cell maturation. Cells 10. https://doi.org/10.3390/cells10082170
    1. Valent P, Akin C, Metcalfe DD (2017) Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood 129:1420–1427. https://doi.org/10.1182/blood-2016-09-731893 - DOI
    1. Valent P, Hartmann K, Bonadonna P, Gulen T, Brockow K, Alvarez-Twose I, Hermine O, Niedoszytko M, Carter MC, Hoermann G, Butterfield JH, Lyons JJ, Sperr WR, Greiner G, Sotlar K, Kluin-Nelemans HC, Schwaab J, Lange M, George TI, Siebenhaar F, Broesby-Olsen S, Jawhar M, Nedoszytko B, Castells M, Orfao A, Gotlib J, Reiter A, Horny HP, Triggiani M, Arock M, Metcalfe DD, Akin C (2022) Global classification of mast cell activation disorders: an ICD-10-CM-adjusted proposal of the ECNM-AIM Consortium. J Allergy Clin Immunol Pract. https://doi.org/10.1016/j.jaip.2022.05.007 - DOI
    1. Valent P, Hartmann K, Bonadonna P, Niedoszytko M, Triggiani M, Arock M, Brockow K (2022) Mast cell activation syndromes: Collegium Internationale Allergologicum update 2022. Int Arch Allergy Immunol:1–13. https://doi.org/10.1159/000524532
    1. Lyons JJ (2021) Inherited and acquired determinants of serum tryptase levels in humans. Ann Allergy Asthma Immunol 127:420–426. https://doi.org/10.1016/j.anai.2021.06.019 - DOI

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